Stable neuropsychological deficits in adult polyglucosan body disease

Greg Savage*, Fiona Ray, Michael Halmagyi, Angela Blazely, Clive Harper

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

10 Citations (Scopus)

Abstract

We describe a 61-year-old woman who gradually developed deficits of balance, gait, and the ability to negotiate movement in space, together with an unusual pattern of cognitive deficits. A series of non-invasive investigations over three years including EEG, CT, MRI, PET and serial neuropsychological review had not provided a diagnosis. Significantly, the four neuropsychological assessments had revealed no progressive decline in cognition. Brain biopsy revealed an abundance of corpora amylacea, and a diagnosis of adult polyglucosan body disease (APBD) was made. This case contributes to the body of knowledge about the cognitive manifestations of this rare disease, and the stability of its functional impact over time.

Original languageEnglish
Pages (from-to)473-477
Number of pages5
JournalJournal of Clinical Neuroscience
Volume14
Issue number5
DOIs
Publication statusPublished - May 2007
Externally publishedYes

Keywords

  • APBD
  • Cerebral biopsy
  • Cognition
  • Neuropsychological assessment

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